抗 IgLON5 病:一个伴有肠梗阻和周围神经病变的病例。

Yunwen Qi, Yongling Zheng, Zhiyou Cai, Xiaogang Zhang
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引用次数: 0

摘要

IgLON5 自身免疫是一种新型抗体介导的疾病,其特征是血清和/或脑脊液(CSF)中的 IgLON5 抗体呈阳性。抗 IgLON5 疾病主要表现为睡眠障碍、运动障碍和脑干综合征。在本研究中,我们报告了一例抗IgLON5病患者,该患者表现为腹胀、腹痛、间歇性排尿困难和便秘,以及间歇性四肢闪电痛,这些症状在抗IgLON5病中并不典型,很容易导致误诊。在进行自身抗体筛查后,我们考虑为抗IgLON5疾病。患者开始接受免疫治疗,包括静脉注射地塞米松、静脉注射免疫球蛋白(IVIG)和口服硫唑嘌呤。治疗后,患者的症状基本缓解。抗 IgLON5 疾病的临床表现多种多样,可能以不同的组合出现,这很容易导致误诊。及早发现并使用免疫抑制剂治疗这种自身免疫性疾病,可能会取得更好的疗效。
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Anti-IgLON5 disease: A case with intestinal obstruction and peripheral neuropathy.

IgLON5 autoimmunity is a novel antibody-mediated disorder characterized by serum and/or cerebrospinal fluid (CSF) positivity for IgLON5 antibody. Anti-IgLON5 disease mainly manifests as sleep disturbances, movement disorders and brainstem syndromes. In this study, we report the case of a patient with anti-IgLON5 disease who presented with abdominal distension, abdominal pain, intermittent dysuria and constipation, and intermittent lightning pain in the extremities, which are atypical of anti-IgLON5 disease and could easily lead to misdiagnosis. After performing autoantibody screening, we considered anti-IgLON5 disease. The patient was started on a course of immunotherapy with intravenous dexamethasone, intravenous immunoglobulin (IVIG) and oral azathioprine (Imuran). Following treatment, the manifestations nearly resolved. The clinical manifestations of anti-IgLON5 disease are diverse and may present in different combinations, which can easily lead to misdiagnosis. Early recognition and treatment of this autoimmune disease with immunosuppressive agents may lead to better outcomes.

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